Prions Flashcards

1
Q

What is a prion

A

A prion is a protein (string of amino acids in specific 3-D shapes.

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2
Q

what is the prion called

A

PrP^c

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3
Q

are PrP living or non-living

A

non-living, no DNA or RNA

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4
Q

Where is PrP found

A

cell membranes

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5
Q

What are the 3 functions of PrP

A
  1. cell to cell communication 2. cell to cell adhesion 3. long term memory
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6
Q

What is the name of the infectious Prion

A

PrP^sc

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7
Q

how does PrP^sc cause disease

A

PrP can be folded wrong, and this wrong folding on the protein causes disease. It has a different structure and is resistant to enzymes in the body that normally break “wrong” proteins. They go around and recruit other normal PrP proteins to cause disease

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8
Q

Explain the term “prions recruit rather than reproduce”

A

The prion acts a template to guide the misfolding of more proteins into prion form. There newly formed prions can then go on to convert more proteins themselves; this triggers a chain reaction that produces large amounts of the prion. This altered structure is extremely stable and accumulates in infected tissue, causing tissue damage and cell death.

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9
Q

What is the incubation period of PrP disease in humans

A

7-40 years

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10
Q

how do mammals get PrP

A

primary method is through ingestion

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11
Q

explain the 3 step process on how prion disease spreads from cow to humans

A
  1. person eats contaminated food. 2. prions spread to the brain through the body’s lymph nodes and immune system where they can lie formant for years 3. disease attacks nervous system. outer layer of brain develops tiny holes
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12
Q

where are prions mostly found in meat

A

primarily found in brain or spinal cord tissue from infected animal

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13
Q

What is the prion disease called in humans, fatality rate?

A

CJD Disease. it is 100% fatal

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14
Q

What is vCJD

A

variant CJD.

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15
Q

differences between CJD and vCJD: cause

A

CJD: health care related (infected instruments), random mutation, cannibalism. vCJD: linked between cattle and humans

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16
Q

difference between CJD and vCJD: prevalence

A

CJD: rate of roughly 1 to 2 cases per 1 million population per year. vCJD: Only 4 reported cases in US

17
Q

difference between CJD and vCJD: symptoms

A

CJD: Dementia; early neurologic signs, Develops much quicker once symptoms start (5 to 6 months)
vCJD: Prominent psychiatric/behavioral symptoms; ; delayed neurologic signs. Symptoms start later (12 14 months)

18
Q

What are two ways to break the chain of infection in target 1 and target 2 (prions)

A
  1. do not ingest meat containing brain or spinal cord products 2. do not engage in cannibalism
19
Q

What is Kuru

A

an incurable degenerative disease endemic to tribal regions of Papua New Guinea. transmitted through cannibalism during funerals

20
Q

What is one way to break chain of infection in target #3 (prions)

A

surveillance of farm animals